Pediatric Hematologic Disorders and Cancer

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Pediatric Hematologic Disorders and Cancer

• Presented by
• Christina Hernandez RN, MSN

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Hematologic System

• Adult

• Pedi

• Life cycle of RBC- 120 days
• Cell production- marrow and spleen
• RBCs 4.1 to 4.9 million/ml
• Hemoglobin
• Hematocrit

• Life cycle of RBC- 100 days (neonate)
• Cell production- red bone marrow (infant)
• RBCs 5million/ml at birth
• Hemoglobin 17-18 g
• Hematocrit 45-50

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Iron Deficiency Anemia
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Iron Deficiency Anemia

• Cause
• Signs and symptoms
• Diagnostic tests
• Nursing interventions
• Oral supplements- What significant side effects
  does the nurse need to remember?
• Dietary teaching- what specific foods?

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What Parents Want to Know (p 1281)

• Specific foods (based on age of child)
• Cream of wheat or iron fortified cereal
• Apricots, prunes, raisins and other dried fruits
• Egg yolks
• Dark green leafy vegetables
• Administration of Iron Supplements
• Give with vitamin C rich fluids
• Prevent staining from liquid iron supplements
• Changes in stool patterns
• Avoid mixing supplement with food/drink
  containing calcium

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Sickle Cell Disease
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Sickle Cell disease

• Sickle cell trait- genetic disorders
  characterized by production of elongated,
  crescent shaped erythrocyte in the place of
  normal Hbg p 1282-1283
• Precipitating factors (p 1284)
• Signs and symptoms

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Three Forms of Sickle Cell Crisis

• Vaso-occlusive
• Acute sequestration
• Aplastic

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Types of Sickle Cell Crisis

• Vaso-occlusive- most common effects
• Pain
• Hand and foot syndrome (dactylitis)
• CVA- hemiplegia, aphasia, seizures, LOC changes,
  vision changes, and headache
• Acute chest syndrome- chest pain, fever cough
  (leading cause of death in SCD)
• Priapism
• Hepatomegaly
• Hematuria

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Types of Sickle Cell Crisis

• Aplastic Crisis
• Decreased RBC production- SS malaise, headache,
  pallor, lethargy, and fainting (precipitated by
  infection)
• Splenic sequestration- life threatening SS
  pallor, irritability, tachycardia, hypovolemic
  shock
• Hyperhemolytic crisis- (not in text)- RBCs
  destroyed more rapidly than usual (immature
  cells)

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Quick Review

• What is most common reason for admission to the
  ED for a child with SCD?
• What precipitates a sickle cell crisis?
• How does sickling effect the life span of an RBC?
• what organs experience complications as a result
  of chronic sickling crisis?

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Diagnosis Treatment

• Cord blood testing if one parent is known to
  carry trait
• Blood transfusions
• Complications
• Nursing interventions before/during/after

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Treatment

• Patient/family teaching
• Medications
• Immunizations- why important?

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Clinical Judgment

• Why are blood transfusions ordered for the
  patient in sickle cell crisis?
• Can a neonate have a diagnosis of sickle cell
  disease?
• What ethical issues relate to this diagnosis?

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Hemophilia
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Hemophilia (p 1291)

• X-linked trait
• What factor is missing or defective?
  Factor VIII
• Who is the carrier, and who is effected by this
  disorder?

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Diagnosis Treatment

• When does diagnosis most commonly occur?
• What specific laboratory tests and values?
• What are signs symptoms?

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Nursing Care

• Factor VIII- when should the patient receive this
  medication?
• What does the family need to know about factor
  VIII?
• Human plasma
• Vasopressin (DDAVP)

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Nursing Care cont

• What is the primary nursing goal for a patient
  with hemophilia?
• Prevent or stop bleeding
• What are specific interventions to achieve this
  goal?
• Administer Factor VIII
• Apply local pressure for 10-15 minutes
• Elevate the joint and immobilize
• Apply cold compresses

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Complications of hemophilia

• Hemarthrosis- assess child for joint pain, edema,
  or permanent deformity. Where most common?
• At risk for hemorrhage
• Death

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The Child with Cancer
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Childhood Cancers
communication promotes understanding and
clarity with understanding, fear diminishes
in the absence of fear, hope emerges and in
the presence of hope, anything is possible
(Stovall, 1995)
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Childhood Cancer

• C- continual unexplained weight loss, fatigue
  malaise
• H- headaches with vomiting (early morning)
• I- increased edema or pain in joints
• L- lump or mass, persistent lymphadenopathy
• D- development of whitish appearance in pupil of
  the eye
• R- recurrent or persistent fevers, night sweats
• E- excessive bruising or bleeding
• N- noticeable pallor

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Acute Lymphatic Leukemia- ALL
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What signs and symptoms would lead to the
diagnosis of leukemia?

• Fever
• Pallor
• Overt signs of bleeding
• Lethargy or malaise
• Anrexia
• Large joint or bone pain
• Petechiae, frank bleeding
• Enlarged liver or spleen, changes in lymph nodes
• Neurologic changes

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Lab values for a diagnosis of leukemia
examination of CBC with at least 25 blasts
confirm the diagnosis

• Normal

• Leukemia

• Leukocytes lt 10,000

• Leukocytesgt 10,000
• Platelets 20-100,000
• Hemoglobin 7-11

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Further diagnostic findings

• Bone marrow aspiration- iliac crest (why this
  site?)
• How does the nurse prepare the child/family for
  this procedure?
• What are the nurse responsibilities for this
  procedure?

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Treatment and Plan of Care (p 1313/1321)

• Chemotherapy three phases
• Induction phase
• Consolidation
• Delayed intensification
• Remission and maintenance

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Nursing Plan of Care for a Child Undergoing
Chemotherapy

• Myelosupression- protect from injury
• Infection/sepsis (neutropenia)- protect from
  infection
• Renal damage
• GI disturbances
• Metabolic emergencies

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Intrathecal Medication

• Chemotherapy instilled into spinal canal
• Assess and monitor for placement of intrathecal
  catheter and assess neuro checks

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Cranial Radiation

• Head and neck tumors are more sensitive to
  radiation than chemotherapy.
• When would chemotherapy become an adjunct to
  radiation therapy?

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Tumor Lysis Syndrome

• What causes tumor lysis syndrome?
• What are signs and symptoms of this complications
• What nursing interventions apply to treatment?

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Bone marrow and Stem Cell Transplantation

• Used to treat leukemia, neuroblastomas and some
  noncancerous conditions-aplastic anemia
• Goal to administer a lethal dose to kill the
  cancer, and resupply the body with stem cells
  from the childs own bone marrow, or a compatible
  donor

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Develop a plan of care for the child undergoing
treatment for leukemia

• Risk for injury
• Soft tissue/mucous membranes
• Generalized trauma
• Risk for infection
• Risk for alteration in bowel elimination
• Risk for GI distress
• Fluid volume delicate

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Other Cancers of Childhood
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Nephroblastoma- Wilms Tumor

• Soft renal tumor - one or both kidneys
• (p 1332) Metastasis or seeding spread by
  palpation
• Nephrectomy treatment of Wilms tumor

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Nursing treatment of Wilms tumor

• Pain management
• Frequent reposition
• Noninvasive and pharmacologic pain interventions
• Prevent circulatory overload
• Weigh daily
• IO, urine for specific gravity
• Prevent infection
• Hand washing
• Protective isolation
• Homecare needs

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Clinical manifestations of Neuroblastoma (p 1327)

• Smooth, hard, non-tender along sympathetic
  nervous system
• Frequent location is abdomen
• Neck and facial edema from vena cava syndrome
• Increased ICP
• Limp if metastasis to bone
• Pancytopenia

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Nursing Management

• Assess by observation and inspection (not
  palpation)
• Document bowel and bladder function
• Record height weight, observe gait
• Chemotherapy, radiation, surgery
• Teach parents SS of infection. Why?

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Osteosarcoma
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Osteosarcoma- most common primary bone malignancy
in children

• Goal of treatment- remove tumor and prevent
  spread of disease
• Biopsy Chemo Surgery Chemo
  (radiationpalliative pain control)
• Promote self esteem
• Side effects of chemotherapy
• Amputation of extremity
• Separation from friends and family

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Ewing Sarcoma- second most common bone tumor
associated with children

• Pain, soft tissue swelling
• Anorexia, fever, malaise with metastasis
• Diagnosis same as osteosarcoma
• Management
• Chemo
• Surgery (decrease tumor bulk)
• Radiation

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Rhabdomyosarcoma
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Pathophysiology and Manifestations

• Most common soft tissue malignancy
• Divided by young (lt10 yrs) and older
  (adolescents) in location
• 60 have positive prognosis
• Soft to hard, nontender mass (depends on
  location)
• In pelvic tumors, may disrupt organ function

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Diagnosis and Treatment

• CT, BM aspiration and biopsy
• Renal function and liver function tests
• Treated with chemo, surgery and radiation

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Retinoblastoma (p1333)
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Retinoblastoma- rare malignant tumor of the
neural retina

• cats eye reflex seen as a white light in the
  pupil is the most common leukocoria
• May have strabismus of involved eye
• Red painful eye is late symptom
• Staging based on extent of disease

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Nursing care of the child/family with a malignant
disease

• Initial focus on support of family members
• Nurses facilitate the educational process to
  allay fears of unknown
• Encourage family members to verbalize fears and
  questions
• Postoperative care if indicated
• Community resources (through the discharge
  planner, case worker)

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Death and Dying

• Understanding of death according to developmental
  age
• lt 3 years- no understanding/concept of death
• 3-5 years- afraid of separation from parents
• 5-9 years- understand death is permanent,
  irreversible and sad. Concerns for fear of pain,
  being left alone and leaving parents and friends.
  
• Age 10gt have adults concept of death

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Nursing Care and Grief

• Child- encourage child to express feelings, allow
  choices, help maintain independence
• Family- listen, answer questions, provide
  information, encourage expression of feelings and
  fears

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• For questions or concerns please contact
• Christina Hernandez RN, MSN
• chernan1_at_austincc.edu
• References
• McKinney, James, Murray, Ashwill. Maternal-
  Child Nursing Third ed (2009). Saunders.